Successful Paediatric Living Donor Liver Transplantation for Biliary Atresia in a 2 Y.O.

Chief Complaints

A 2-year-old baby boy was brought to the Liver Transplant Department at PACE Hospitals, Hitech City, Hyderabad, for advanced treatment of biliary atresia. The child had progressive jaundice despite undergoing a Kasai procedure during infancy and had subsequently developed decompensated chronic liver disease.

The child was a known case of biliary atresia with decompensated chronic liver disease and progressive jaundice. He had previously undergone a Kasai portoenterostomy at four months of age; however, the procedure did not provide adequate long-term bile drainage, and the liver disease continued to progress. No other significant medical comorbidities or previous surgical procedures were reported. There was no documented history of medication or food allergies.

On examination, the child was conscious and clinically stable, with visible jaundice and findings suggestive of chronic liver disease. Abdominal examination showed mild distension with minimal fluid accumulation. No signs of acute abdominal tenderness or active bleeding were noted. The child was further assessed for nutritional status, respiratory condition, signs of infection, portal hypertension, and overall fitness for liver transplantation.

Upon admission to PACE Hospitals, following a detailed clinical assessment, the Surgical Gastroenterology and Liver Transplant team evaluated the child for progressive jaundice following a previous Kasai procedure, along with a review of his relevant past medical history.

Clinical evaluation and review of the patient's medical history revealed findings consistent with biliary atresia with decompensated chronic liver disease following a failed Kasai procedure. The diagnosis was established based on the history of persistent jaundice after Kasai portoenterostomy, clinical features of progressive liver dysfunction, and assessment of the Paediatric End-Stage Liver Disease (PELD) score.

Routine investigations, including complete blood picture, liver function tests, coagulation profile, renal function tests, liver biopsy, and other pre-transplant investigations, were performed as part of the preoperative evaluation to assess the severity of liver disease, transplant suitability, and the child's overall medical condition. These investigations supported the diagnosis and helped in planning the liver transplantation procedure.

Based on the confirmed diagnosis, the child was advised to undergo Biliary Atresia Treatment in Hyderabad, India, under the expert care of the Liver Transplant Department at PACE Hospitals.

After a detailed consultation with Dr. C. H. Madhusudhan (Senior Consultant Surgical Gastroenterologist and Liver Transplant Surgeon), a comprehensive evaluation was performed to determine the most appropriate therapeutic approach.

Considering the child’s history of biliary atresia, previous Kasai procedure, progressive jaundice, decompensated chronic liver disease, and a Paediatric End-Stage Liver Disease score of 19, further evaluation confirmed advanced liver damage with inadequate response to the earlier surgical treatment. Preoperative investigations, including liver function tests, liver biopsy, complete blood count, platelet count, and other transplant-related assessments, were reviewed to determine the child’s suitability for surgery.

Based on the clinical assessment and transplant evaluation, it was determined that Living Donor Liver Transplantation was the most appropriate and effective management strategy. This approach was chosen to replace the severely damaged liver, restore liver function, improve bile drainage, prevent further progression of liver failure, and provide better long-term survival and quality of life.

The child’s family and the proposed donor were counselled regarding the diagnosis, clinical findings, planned liver transplantation procedure, perioperative risks, possible postoperative complications, the need for immunosuppressive treatment, and the importance of regular follow-up.

Following the decision, the patient was scheduled for Living Donor Liver Transplantation in Hyderabad at PACE Hospitals, under the care of the liver transplant team.

The following steps were carried out during the Living Donor Liver Transplantation:

• Patient Preparation and Anaesthesia: After completing the required preoperative investigations, donor evaluation, transplant clearances, and informed consent, the child was taken up for surgery. General anaesthesia was administered, and continuous monitoring was maintained throughout the procedure.

• Abdominal Exploration and Adhesiolysis: The abdomen was opened and carefully examined. Macronodular cirrhotic changes, minimal ascites, multiple abdominal adhesions, enlarged lymph nodes, and a hypoplastic portal vein were noted. The adhesions from the previous Kasai procedure were carefully released.

• Removal of Diseased Liver: The previously created portoenterostomy was dismantled, and the damaged cirrhotic liver was carefully separated from the surrounding structures. The diseased liver was removed while preserving the required blood vessels for graft implantation.

• Donor Liver Graft Implantation: A suitable portion of the liver donated by the child’s maternal uncle was implanted. The hepatic veins, portal vein, hepatic artery, and bile drainage pathway were reconstructed to restore blood supply and bile flow through the transplanted liver.

• Doppler Assessment and Abdominal Closure: Doppler examination was performed to confirm normal blood flow through the transplanted liver. The abdominal wall was closed with the support of Vicryl mesh, and a repeat Doppler assessment after skin closure showed normal findings.
  

The child was closely monitored for graft function, blood flow, infection, breathing difficulty, and overall recovery. Pleural effusion and abdominal fluid collections were managed with image-guided drainage, while respiratory support was provided when needed. Treatment was given to prevent rejection, control infection, maintain hydration, relieve pain and fever, protect the stomach, and support nutrition. Liver function improved gradually, and the child was discharged in a stable condition.

At discharge, the child was advised to continue medicines to prevent rejection of the transplanted liver, reduce the risk of infection, protect the stomach, control pain or fever when required, support nutrition, and manage any remaining postoperative symptoms. The dosage was adjusted according to liver function and clinical response. The family was instructed to give all medicines exactly as prescribed and not to stop or change them without consulting the liver transplant team.

The family was advised to give all prescribed medicines at the correct time, maintain proper hygiene, and keep the surgical wound clean and dry. The child should be given nutritious food and adequate fluids as advised, while avoiding exposure to people with fever, cough, cold, or other infections.

The family was informed to contact the emergency ward at PACE Hospitals in case of any emergency or development of symptoms such as fever, abdominal pain, vomiting, breathing difficulty, increasing jaundice, abdominal swelling, reduced urine output, poor feeding, unusual drowsiness, bleeding, or any discharge from the surgical wound.

The patient was advised to return for a follow-up visit with the Surgical Gastroenterologist in Hyderabad at PACE Hospitals after 3 days.

This case highlights the successful management of biliary atresia with decompensated chronic liver disease following an unsuccessful Kasai procedure. Living Donor Liver Transplantation was performed using a liver graft donated by the child’s maternal uncle. Postoperative complications were identified and managed appropriately with intensive monitoring and supportive care. The child showed improvement in liver function and was discharged in a hemodynamically stable condition.